Tics in Childhood Tics, Tourette’s Rationale and DSM V � Tics are the most common movement disorder seen in childhood. Thomas K. Koch, MD � Primary care providers are often reluctant to Credit Unions for Kids diagnose Tourette’s without neurologic consultation. Professor of Pediatric Neurology � Proper evaluation and recognition of the true tic burden and any associated co-morbidities is essential for proper treatment. � Counseling and treatment discussions are critical in the management of these patients and families. Tics, Tourette’s Tics in Childhood and DSM V Objectives Presented by Thomas K. Koch, MD Faculty Disclosure � Recognize and classify the common variety of tics Information seen in children A. I do not have any financial relationships with the manufactures of � Be comfortable with making the diagnosis of any commercial product and/or provider of commercial services Tourette Syndrome discussed in this CME activity: � Be able to properly discuss both the natural history of tics and Tourette as well as B. I do intend to discuss an unapproved / investigative use of a pharmacologic and non-pharmocologic treatment commercial product / device in my presentation. options.
Tic Classification Tics in Childhood Features Simple Complex Eye blinking Facial grimacing � Common: 4% to 24% of all children Head twitching Touching � Most common movement disorder Head thrusting Smelling Motor Shoulder shrugging Jumping � Involuntary stereotypic repetitive Mouth opening Echokinesis Copropraxia movements or vocalizations � May be transient or chronic Sniffing Echolalia Snorting Palilalia Vocal Coughing Coprolalia Throat clearing Grunting Barking Tics in Childhood Characteristics � Wax and Wane � Exacerbated by stress, excitement, anxiety, fatigue � Improve with rest, relaxation, concentration � Usually absent during sleep but may be present on polysomnograms � Briefly suppressible; build up of “inner tension” � Often preceded by a premonitory urge or “sensory tic”
Tic Syndromes in Children Tic Syndromes in Children � Provisional Tic Disorder (DSM V) � Chronic Tic Disorders (>1yr) � Chronic Tic Disorder � Chronic Multiple Motor or Vocal Tics � Chronic Motor or Vocal Tics � Tourette Syndrome � Tourette Syndrome � Nonspecific Tic Disorder In 1885, George Gilles de la Tourette reported nine patients with chronic tic � Secondary to Drugs (Stimulants) disorders characterised by involuntary � Assoc with Autistic Spectrum motor and phonic tics. � PANDAS ? Tourette Syndrome Tic Syndromes in Children Criteria (DSM V) � Provisional Tic Disorder � Onset < 18 yrs of age � Most common � Multiple motor tics � Duration < 1 year � One or more vocal tic � Single motor or vocal tic � A waxing and waning course � Rx usually not necessary � Duration > 1 yr � Absence of medical explanation for tics DMS V, 2013
Diagnostic Criteria Tourette syndrome 1 Tourette disorder 2 Onset : By age 21 By age 18 Motor tics: multiple Vocal tic at least one Course: gradual; wax & wane Duration: > 1 year Medications: no tic provoking medications Other: not due to other disease Witnessed: Observed 1 TS Classification Group 1993 2 DSM V Tourette Syndrome Tourette Syndrome Clinical Course of Tics Clinical Facts � Worldwide distribution � Wax and Wane � Prevalence: 1 per 1000 up to 3.5% of school age � Maximum severity between 8-12 yrs � Inherited but probably more than one gene � Early severity = Later severity � 3:1 male > female � Prognosis: Most improve � Onset: 6-7 yrs (mean) � 26% resolved � Usually before adolescence � Usually begins with simple motor tic 72% Improve � 46% diminished � Increase with stress and anxiety � 14% stable � Examination � 14% increased � Tics � “Soft signs” Leckman JF et al. Pediatrics, 1998 Erenberg et al. Ann Neurol, 1987
Tourette Syndrome Tourette Syndrome Comorbidity Comorbidity � Obsessive Compulsive Disorders – 20-89% � Attention Deficit-Hyperactive Disorder � Attention Deficit Hyperactive Disorder – 50% � 50-60% of TS patients (21-90%) � Anxiety – 19-80% � Generally begins before tics by 2-3 yrs � Mood Disorders - Depression – 30-40% � Not associated with the tic severity � Learning Difficulties – 20-30% � Characterized by: � Other � Impulsivity / Hyperactivity � Impulsivity and aggression � Poor attention � Substance Abuse Tourette Syndrome Tourette Syndrome Comorbidity Genetic Epidemiology � Obsessive-Compulsive Behaviors � Overall risk of TS in relatives is 10.7% � 20-89% of TS patients � Male relatives – 17.7% � Female relatives – 5.2% � Usually emerge after tics � Concordance rate for TS � Usually obsessions or compulsions � MZ twins – 86% � Associated with: � DZ twins - 20% � Impulsivity / Aggression � Depression / Anxiety
Tourette’s Syndromes Tourette’s and the SLITRK1 gene Neurobiology � Cortico-striatal-thalamocortical pathway � SLITRK1 is a Tourette gene � Neurotransmitters � Only accounts for < 2% of TS patients � Dopamine � Other candidate genes: � GABA � Chromosome 17 � Glutamate � Chromosome 8 � Noradrenergic � Chromosome 2 � Serotonin � Chromosome 11 � Cholinergic � Opioid Abelson JF, et al. Sequence variants in SLITRK1 are associated with Tourette’s syndrome. Science 2005;310:1-9. What is the risk to my children if Tourette’s Syndromes I have Tourette ? How to Rx – What to Rx � Risk for TS – 10% Tics � Risk for a tic disorder – 30% � Risk for OCD – 30% � Risk for ADHD – 40% � Risk for any of the three – 60% � Higher risk if both parents have TS � 75% for a tic disorder � 50% for Tourette ADHD OCD � 95% for any of above
Tic Treatments Habit Reversal Therapy Options � Habit reversal training consists of two main components. These are: � Education *** � Tic-awareness training, which teaches patients to � Behavioral approaches recognize early signs that precede the onset of a tic � Competing-response training, which teaches patients to � Pharmacotherapy perform a voluntary movement that is incompatible with the particular type of tic � Deep brain stimulation JAMA. 2010;303(19):1929-1937. Tic Treatment Behavior therapy for children with Tourette disorder: a randomized controlled trial. Non-pharmacologic Therapy Piacentini J, Woods DW, Scahill L, Wilhelm S, Peterson AL, Chang S, Ginsburg GS, Deckersbach T, Dziura J, Levi-Pearl S, Walkup JT. Design, Setting, and Participants: Randomized, observer-blind, controlled trial of 126 children recruited from December 2004 through May 2007 and aged 9 through 17 years, with impairing Tourette � Relaxation therapy or chronic tic disorder as a primary diagnosis, randomly assigned to 8 sessions during 10 weeks of behavior therapy (n = 61) or a control treatment consisting of supportive therapy and education � Habit reversal training (n = 65). Responders received 3 monthly booster treatment sessions and were reassessed at 3 and 6 months following treatment. � Acupuncture Main Outcome Measures: Yale Global Tic Severity Scale (range 0-50, score >15 indicating clinically significant tics) and Clinical Global Impressions–Improvement Scale (range 1 [very much improved] to 8 [very much worse]). � Biofeedback Results: Behavioral intervention led to a significantly greater decrease on the Yale Global Tic Severity Scale (24.7 [95% confidence interval, 23.1-26.3] to 17.1 [95% CI, 15.1-19.1]) from baseline to end point � Hypnosis compared with the control treatment (24.6 [95% CI, 23.2-26.0] to 21.1 [95% CI, 19.2-23.0]) ( P < .001; difference between groups, 4.1; 95% CI, 2.0-6.2). Significantly more children receiving behavioral intervention compared with those in the control group were rated as being very much improved or much improved on the Clinical Global Impressions–Improvement scale (52.5% vs 18.5%, respectively; P < .001; number needed to treat = 3). Attrition was low (12/126, or 9.5%); tic worsening was reported by 4% of children (5/126). Treatment gains were durable, with 87% of available responders to behavior therapy exhibiting continued benefit 6 months following treatment.
Tic Treatment Tic Treatment Pharmacotherapy – Non-neuroleptics Pharmacotherapy – Others Dose Dose � Botulinum toxin Generic Brand Starting (mg/d) Usual (mg/d) � delta-9-tetrahydrocannabinol Clonidine Catapres 0.025-0.05 0.1-0.3 � Nicotine patch Guanfacine Tenex 0.25-0.5 0.5-3.0 � Tetrabenazine � Ropinirole Baclofen Lioresal 10-15 20-60 Clonazepam Klonopin 0.025-0.5 0.5-3.0 Topiramate Topamax 15-25 100 Tic Treatment Practical Points Pharmacotherapy – Neuroleptics ■ Chronic tics are common Dose Starting Dose Usual ■ Do not assume tics are cause of disability (mg/d) (mg/d) Generic Brand ■ Ascertain comorbidities, and identify impairment/disability Pimozide Orap 0.5-1 1-10 ■ Pharmacologic therapy for tics should not be the default Risperidone Risperdal 0.25-0.5 0.5-3.0 ■ Relatively low impact strategies are often sufficient Fluphenazine Prolixin 0.25-1.0 0.5-6 ■ Education, stress reduction ■ Cognitive/behavioral intervention Olanzapine Zyprexa 2.5 2.5-10 ■ Beware the early institution of neuroleptics Haloperidol Haldol 0.25-0.5 1-5 ■ Therapy for comorbidities may help to ameliorate tics
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